Differences in the pathophysiology of hemolysis of alpha- and beta-thalassemic red blood cells.
The basic pathology in all forms of thalassemia results from the presence of excess unstable globin chains within the pathological RBC, but the pattern and rate of their precipitation is different. Consequently, their effects on the RBC membrane components are not the same and may account for the different rheological properties that have been found. It is possible that the damage incurred by excess beta chains in Hb H disease is primarily due to the direct interaction of the large inclusions with some cytoskeletal proteins such as spectrin, ankyrin, and band 3. In beta-thalassemia, where excess unstable alpha chains have already precipitated in young erythroblasts, the main damage might be caused by an excess of free oxygen radicals, which affect in particular protein 4.1. A search for additional changes and for potential differences in the membrane and cellular properties between the different thalassemic syndromes is warranted in order to understand better the different clinical expression in the various types of the disease. Moreover, when there is a better elucidation of the mechanisms by which the RBC are destroyed, one may look for possible ways and means to prevent these changes, with a consequent extension of the current short life span of the affected RBC.
Shinar
E
E
Magen David Adom Blood Services Center, Israel.
Rachmilewitz
E A
EA
D004910:Erythrocyte Membrane / Q000648:ultrastructure; D004912:Erythrocytes / Q000473:pathology / Q000502:physiology* / Q000648:ultrastructure; D005914:Globins / Q000378:metabolism; D006801:Humans; D013789:Thalassemia / Q000097:blood*
D005914:Globins